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NEUROLOGY 1998;50:1166-1169
© 1998 American Academy of Neurology

Heritable connective tissue disorders in cervical artery dissections: A prospective study

W. I. Schievink, MD, E.F.M. Wijdicks, MD, V. V. Michels, MD, J. Vockley, MD, PhD and M. Godfrey, MD

From the Departments of Neurologic Surgery (Dr. Schievink), Neurology (Dr. Wijdicks), and Medical Genetics (Drs. Michels and Vockley), Mayo Clinic, Rochester, MN; and Department of Pediatrics and Munroe Center for Human Genetics (Dr. Godfrey), University of Nebraska Medical Center, Omaha, NE.

Address correspondence and reprint requests to Dr. Wouter I. Schievink, Barrow Neurological Institute, 350 West Thomas Road, Phoenix, AZ 85013.

We prospectively evaluated 15 consecutive patients with spontaneous cervical artery dissections. Three patients (20%) had a heritable connective tissue disorder, each with a unique phenotype. None of these patients met the criteria of any of the named syndromes, and collagen and fibrillin analyses were normal. Heritable connective tissue disorders are common among patients with spontaneous cervical artery dissections, but, despite intensive investigations, the type of disorder usually cannot be identified. The underlying arteriopathy in cervical artery dissections is likely to be heterogenous.

Received July 11, 1997. Accepted in final form September 18, 1997.






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